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Malignant haematology > Acute lymphoblastic leukaemoa - see paeds? > Flashcards

Acute lymphoblastic leukaemoa - see paeds? Flashcards

1
Q

What is acute lymphoblastic leukaemia

A

a malignancy of precursor cells from the lymphocyte lineage, the most common of which is B-cell
Most common childhood malignancy

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2
Q

Red flags for ALL

A

Persistent fever
Pallor
unusual bleeding
Lymphadenopathy
Organomegaly

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3
Q

Cause of ALL

A

Genetic - indrease risk among monozygotic twins, trisomy 21, kleinfelter syndrome and fanconi anemai
Viral exposure
Smoking or radiation exposure

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4
Q

Pathophysiology ALL

A

Precursor cells chromosomal mutations arrest development -> continue clonalm expansion and more mutations -> uncontrolled proliferation of lymphoblasts -> bone marrow and organ infiltration, restrict haematopoiesis and supress bone marrow

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5
Q

ALL classifications

A

B cell lineage
T cell lineage
Rare cases NK cell lineage

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6
Q

Why use cytogenetic and molecular testing in B-ALL when symtpoms the same

A

Different subtypes have different risk - stratify risk and determine agression of treatment use
Prognostic

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7
Q

Examples of ALL classification

A

Philadelphia chromosome
Hyperdiploid - B-ALL/LBL
Hypodiploid B-ALL/LBL
T(v11q23.3) KMT2A rearranged

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8
Q

Clinical features ALL

A

Hepatomegay or spenomegaly ->anorexia, weight loss, abdo pain, distension
Fever - persistent, recurrent or refractort, night sweats, weight loss
Lymphadenopathy
Haematological = unusual bleed or petechial rahs, anaemia
MSK pain - bone pain - limp, refusal to weight bear
All present around 50% cases

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9
Q

Lymphadenopathy in ALL

A

Persistent or progressive painless firm rubbery ,ymph node

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10
Q

Less common clinical features ALL

A

Headache - ICP, N+V, irritability, meningism or focal neuro deficits
Testicular enlargement - more likely in relapse
Mediastinal mass - T cell lineage -> SVCc compression

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11
Q

What can present with SVC compression and what does that looj like

A

T cell lineage ALL due to mediastinal mass
Dysphagia, SOB, oain, swelling of face and upper limbs

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12
Q

FBC in ALL

A

Thrombocytpoenia
Anaemia
WCC - low (50%), high (20%), normal if bone marrow not supressed yet
Depends if arrested post or pre maturation
Neutropenia most likely

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13
Q

What investifations are required in ALL to screen for complications

A

Infection screen
Coag profile
U+Es, LDH, uric acid

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14
Q

ALL further investigations

A

Peripheral blood smear
Bone marrow aspiration

Can do - lymph node biopsy, CSF w LP

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15
Q

What investigations need to diagnose ALL

A

Immunophenotyping and cell morphology

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16
Q

When do lymph node biopsy in ALL

A

lymphadenopathy presenting feature - easier to see abnormal cells

17
Q

What do all children with ALL have due to poor CNS involvement prognosis

A

CSF to evaluate for involvement
Prophylactic intrathecal chemo

18
Q

What are the potential problems with a peripheral smear

A

May be normal if malignancy confined to bone marrow on presnetation

19
Q

What use cytogenetics for in ALL

A

Classification of ALL to subtype WHP

20
Q

Differentials for ALL

A

Burkitts lymphoma
AML
Aplastic anaemia
ITP
Reactive lymphocytosis

21
Q

ALL vs AML

A

Clinically and morphologically indistinguishabel
EXCEPT ALL -> testicular and CNS involvement
AML -> skin and gum or mucous membranes
Use cytogenetics and immunophenotyping of bone marrow to confim
AML - meyloid features and antigens and absence of lymphoid antiens

22
Q

Aplastic anaemia differentiate ALL

A

Absence of blast cells

23
Q

Waht is reactive lymphoytosis

A

Non specific features similar to ALL
seroloy and iral tests, cultures and normal haematopoiesis
eg infectious monoculeosis
Pertussis
HIV
osteomyelitis
TB

24
Q

Inital management ALL prior to starting therapy

A

Treat any infection, metabolic complication and transfusion if indicated
5-7 dyas of corticosteroids
hYDRATION
aLLOPUIRONAL
CNS prophylacis intrathecal
Baseline U+Es, LFTs, ECHO function

25
Q

Which chemo is cardiotoxic in heam

A

Anthracycline

26
Q

Complications of ALL

A

Pancytypenia (TP, anaemia, neutropenia (sepsis))
Leukostasis >100 lymphoblasts

27
Q

What need to regularaly monitor in ALL

A

FBC - identify low levels and need for correction or prophylaxic antibiotics

28
Q

Treatment leukostasis

A

Leukopheresis

29
Q

ALL complications from therapy

A

Tumour lysis syndrome - induction therapy
GI toxicity - nause, mucositis, diarrhoea, const, abdo pain
Coticosteroid -related avascular necrosis
L-asparaginase-ralted coagulopathy
Anthracycline related cardiotoxicity

30
Q

What chemo drug can cause coagulopathy

A

L-asparaginase
pFibrinogen and anti-thrombin 3 depletion

31
Q

Factors -> improved prognosis in ALL

A

Younger patients >adults
Low WCC > high at diagnosis
T-cell worse than B-cell
Chromosomal abnormalities negatively affect - Ph+, IKFZ1

32
Q
A

Malignant haematology (16 decks)

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